Living with microcephaly: A story of two sisters, The Hartley Hooligans

Mrs Gwen Hartley with her daughters, who have microcephaly.
Mrs Gwen Hartley with her daughters, who have microcephaly.PHOTO: THEHARTLEYHOOLIGANS.COM

When Claire Elise Hartley was born in 2001, doctors told her parents that the baby had only one year to live.

Claire has microcephaly, a condition in which a baby is born with an abnormally small head and brain.

"They told me that she would not live to be one. They told me she was 'not in there'... They said she would die of pneumonia. I bawled," said Mrs Gwen Hartley, 41, who is from Kansas in the United States.

But Claire survived, even though she also suffers from dwarfism, cerebral palsy, epilepsy and scoliosis.

In 2006, Mrs Hartley had a second daughter, Lola Grace.

Lola, too, has microcephaly, as well as dwarfism, epilepsy and other conditions which have severely affected her development.

"We were floored that it happened a second time. It was very hard to adjust to the fact that we'd be the parents of not one, but two special needs children. And we were terrified of losing both of them," she told The Straits Times.

For Claire and Lola, the cause of microcephaly is genetic.


Claire Elise Hartley was born in 2001. Doctors told her parents that she had only one year to live. PHOTO: THEHARTLEYHOOLIGANS.COM

Both Ms Hartley and her husband are believed to carry a rare gene which caused it. The chance of the condition occurring in their children is 25 per cent. They have an older son Cal, 17, a straight-A student and basketballer.

But for up to 4,000 babies in Brazil, microcephaly is said to be linked to the Zika virus, which is carried by the Aedes mosquito.

 
 
 

While researchers have not found a definitive link between Zika and fetal brain damage, reported cases of microcephaly have risen sharply in Brazil, ground zero for Zika.

The virus has since spread to more than 20 countries in Latin America. On Feb 1, the World Health Organisation declared the virus an international public health emergency.

Not all babies diagnosed with microcephaly have intellectual disabilities. About 10 per cent have normal intelligence, some never walk or talk, while many have disabilities but are high-functioning.

Experts say the prognosis for the child can vary widely depending on the cause of the microcephaly, which can also be a result of a premature fusing of the joints between the bony plates that form an infant's skull and decreased oxygen to the fetal brain.

Other causes include infections like German measles and chicken pox, exposure to drugs, alcohol or toxins, and severe malnutrition.

Reports of cases in Brazil indicate that many of the children are severely affected. They suffer a large degree of brain tissue loss, have smooth brains and smaller cerebellums, which play a role in motor control, The New York Times reported.

The outbreak has thrown the spotlight on what it is like living with microcephaly, for both the sufferers and their caregivers.

"I am sad that so many babies are being born with microcephaly, but they are the cutest little babies I've ever seen... My hope is that support can come to those families whose babies are affected," Mrs Hartley said when asked about the cases in Brazil.

BEATING THE ODDS


The Hartley family in November last year. PHOTO: THEHARTLEYHOOLIGANS.COM

In an e-mail interview with The Straits Times, she spoke of the joy, and pain, of living with two children affected by the condition.

For children with such profound disabilities, her daughters have beaten the odds.

Claire is now 14 and Lola is nine.

Neither can walk, talk or do many of the things other children take for granted, but they have also led longer and healthier lives than doctors thought they would.

Developmentally, both are more like babies than girls their age. Intellectually, they are possibly "6-18 months", said Mrs Hartley. Both are small - Claire is the size of a toddler (about 10kg) and Lola a three-month-old baby (5.4kg).

For the two sisters, everything from eating, moving, grasping things and making eye contact has been difficult.

"We cherish things like eye contact, grasping our finger, smiles, weight bearing through their legs, head control - everything we took for granted with our son," she said.

The two girls require 24-hour care. The sleep deprivation that new mothers go through is something she has experienced for more than a decade.

To go grocery shopping, the Kansas mum needs to arrange for a baby sitter; to get a full night's rest, they have engaged a night nurse for the past few years; and to go anywhere with her two daughters requires both parents.

But the feisty mum and former teacher still finds time to write about the daily travails of their family on her blog, The Hartley Hooligans.

While other parents look forward to their children's first steps or words, she had to fight for Claire and Lola to stay alive.

She recalled that when Claire was born, the doctor told her and her husband Scott to sign a Do Not Resuscitate (DNR) form so that when the child died, she could "just let her go".

She ignored the doctor's instructions and chose not to put her daughter on a host of recommended medications.

"We began feeding her an organic diet, using natural supplements, we did energy medicine and homeopathics... We use no medications with them at all. I am not saying this is the answer for every child, but for our daughters, this natural route... feels right."

The girls also go through a gamut of therapies, with a physical therapist, an occupational therapist, a vision teacher and a speech and technology teacher visiting the Hartley home every week.

The special equipment they need for daily life is, in their mum's words "out of control".

"I am blown away by the sheer cost of the equipment needed to simplify my girls' lives," she said. "The bath chairs, sitters, standers, stander/sitters, walkers, scales, wheelchairs, double wheelchairs."

For the Hartleys, much of this is paid for with private and public health insurance, and help from special needs organisations.

Mr Hartley, formerly a teacher, now works as a glass blower. He works six days a week but is still very involved with caring for their children, she said.

"When he comes home, no matter how tired he is, he immediately steps in and helps care for the girls."

Despite the enormous changes they have had to make, Mrs Hartley said she thrives on Claire and Lola's "little accomplishments" and the kindness that strangers have shown to them have "restored her faith in humanity".

Claire is learning to sit, kneel and stand, and is now moving her hand to communicate. Lola is "more visually attentive", and is using her nose to answer yes or no questions.

On her blog, an entry from Halloween 2015 shows the family in superhero gear. Claire is dressed in a Wonder Woman outfit, while the diminutive Lola was a pint-sized Incredible Hulk. Their brother Cal is Superman.


The family dressed up in superhero gear for Halloween last year. PHOTO: COURTESY OF GWEN HARTLEY

Referring to her children as "tiny superheroes", she wrote: "Any child who has gone through something beyond difficult and come out the other side even stronger is a hero in my book ."

For Mrs Hartley, who remembers how anxious she felt during Claire's first year, the prospect of losing her two daughters is far more daunting than living with their conditions. She has almost 17,000 photos on her smartphone, all backed up.

"I cannot bear to delete pictures of my kids. I never want to forget them," she said.

In her reply to The Straits Times, she said they have learnt to live "in the now" and to take less for granted.

"I remember thinking after we had Claire that we'd have the 'perfect' family because we'd have this new house, a boy, a girl and a couple of dogs, and life would be exactly what we planned. After having Claire, our focus definitely changed. But our family was still perfect."